Sclerosing mesenteritis

Other Names: Retractile mesenteritis; Liposclerotic mesenteritis; Mesenteric lipogranuloma; Mesenteric fibromatosis; Mesenteric panniculitis; Mesenteric lipodystrophy; Peritoneal retractile mesenteritis; Idiopathic sclerosing mesenteritis

Sclerosing mesenteritis is one of many terms that describes a spectrum of inflammatory disorders that affect the mesentery. The mesentery is the membrane that anchors the small intestine to the back of the abdominal wall.

The condition mostly affects men between their 40s and 60s, but women and children can also be affected.

Epidemiology

Although described as a rare disease, it is thought that the incidence of sclerosing mesenteritis could be as high as 3.4%, with a reported range of 0.16–3.4% . This incidence is difficult to determine as many patients diagnosed initially with primary sclerosing mesenteritis are found to have secondary sclerosing mesenteritis usually due to underlying malignancy. In a prospective evaluation of 7,000 consecutive abdominal computed tomography (CT) scans, Daskalogiannak reported an incidence of about 0.6%. Sclerosing mesenteritis is commonly diagnosed in the fifth to seventh decade of life although a case report with a three-year-old patient has also been published . A recent systematic review of 192 cases of sclerosing mesenteritis showed an age range of 3–88 years with a mean age of 55 +/− 19.2 years . Most of the studies have been performed on the Caucasian population which limits generalization across the multiple ethnicities.

Cause

The cause of the condition is poorly understood. Suggested possible causes have included previous abdominal surgery or trauma, autoimmunity,paraneoplastic syndromes, ischemic injury, and infection.

Inheritance

To our knowledge, sclerosing mesenteritis is not a hereditary condition. Studies suggest that up to 1 in 100 people may have sclerosing mesenteritis, suggesting that many cases are never diagnosed. While the exact cause of the condition is not known, the following factors have been suggested to possibly play a role in causing the condition in some cases:

• A history of abdominal surgery or trauma
• Having an autoimmune condition, including Riedel thyroiditis, primary sclerosing cholangitis, retroperitoneal fibrosis, and orbital pseudotumor, autoimmune hemolytic anemia, minimal change nephropathy, systemic lupus erythematosus, relapsing polychondritis, limited systemic sclerosis, or having elevated IgG4 in the blood and/or autoimmune pancreatitis
• Having an underlying cancer (paraneoplastic syndrome)
• History of infection

Symptoms

Sclerosing mesenteritis may cause a variety of gastrointestinal and systemic symptoms, such as:

• Abdominal pain
• Nausea and vomiting
• Diarrhea or constipation
• Rectal bleeding
• Weight loss
• Abdominal distention (bloating)
• Fever
• A palpable abdominal mass

Reports in the literature have indicated that symptoms can last anywhere from days to years. In severe cases, the symptoms can have a significant effect on quality of life. Some individuals develop complications such as a bowel blockage.

Diagnosis

There is no specific laboratory biomarker/test available for the diagnosis of sclerosing mesenteritis although the elevation in inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) has been reported in as high as in 80% of cases .

Imaging characteristics Abdominal imaging is the mainstay for the diagnosis of the sclerosing mesenteritis, and the advent of CT scan has drastically improved the diagnostic incidence of sclerosing mesenteritis. Since 1924 when the sclerosing mesenteritis was first recognized and especially after 1980 the number of case reports and case series have dramatically increased .

Sclerosing mesenteritis can be visualized on abdominal ultrasound, CT scan, and MRI, but CT scan is the most utilized imaging modality recorded in the literature . However, in the initially published literature, barium radiographs of the abdomen were also utilized, but it was useful only for severe cases where significant external compression of the bowel had already occurred distorting the underlying anatomy .

One of the specific CT scan signs to diagnosis sclerosing mesenteritis is a “fat ring sign” but presence of “pseudo-capsule” also raise the suspicion of sclerosing mesenteritis. Infrequently, a sign of “misty mesentery” has also been described which is an increased in mesenteric attenuation with the presence of small nodes in the absence of a discrete soft-tissue mass suggestive of sclerosing mesenteritis .

In the year 2011, Coulier published a five-sign CT imaging criteria specifically for mesenteric panniculitis. These criteria were later accepted by many authors and subsequently were used in many other studies for the diagnosis of sclerosing mesenteritis .

These five diagnostic signs included the presence of a well-defined “mass effect” on neighboring structures (sign 1) constituted by mesenteric fat tissue of inhomogeneous higher attenuation than adjacent retroperitoneal or meso-colonic fat (sign 2) and containing small soft tissue nodes (sign 3). It may typically be surrounded by a hypo-attenuated fatty “halo sign” (sign 4) and an hyperattenuating pseudo-capsule may also surround the entity (sign 5). However, these diagnostic criteria were not histologically verified, but they have overall come to represent the closest general standards available .

Treatment

There is no standard therapy for sclerosing mesenteritis, and treatment is based on the severity and specific symptoms in each person. Several medications have been used to try to stabilize the condition either alone or in combination. However, use of most of these medications are based on case reports or small case series. These have included:

• Glucocorticoids (prednisone)
• Hormonal therapy (tamoxifen and progesterone)
• Low dose naltrexone
• Colchicine
• Cyclophosphamide
• Thalidomide
• Surgery is typically reserved for obstructive complications (bowel obstruction). In some cases, the condition goes away on its own.

NIH genetic and rare disease info

• NIH info on Sclerosing mesenteritis

Sclerosing mesenteritis is a rare disease.

Sclerosing mesenteritis Resources
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